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Washington University Experience | NEURODEGENERATION | Pick Disease | 5A0 Case 5 History
Case 5 History ---- This patient was initially evaluated at the ADRC at the age of 63. He had 4 years of Primary Progressive Aphasia and Semantic Dementia symptoms which were initially noted to be repetition of conversations, misplacing items, difficulty calculating tips, and difficulty operating a new DVR. By 2012, formal neuropsychology testing was notable for deficits in semantic memory, moderate naming deficits, mild deficits in executive function, anterograde memory and spatial ability leading to the diagnosis of FTD/ semantic dementia, CDR 0.5. By 2013, formal neuropsychology testing revealed a decline in verbal anterograde memory, naming, and problem solving, leading to the diagnosis of semantic dementia (FTD) of mild to moderate severity (CDR 0.5). One year later on the MAP aphasia battery, he was noted to have errors in oral naming and reading comprehension. Overall, he was noted to have impaired memory, profoundly impaired Boston naming, impaired animal and letter fluency, and decreased comprehension with repetition. Neuro exam was notable for L>R agraphesthesia. He retired from his profession due to issues with memory, language, and mood. In 2017: CDR was 2 early in the year to CDR 3 with Boston naming 1 towards the end of the year. In 2021 he died after an 11-year history of slowly progressive cognitive decline consistent with FTD that started as semantic dementia. In 2021 CDR was 3. He died in a nursing home 8 months later after a brief GI illness with a distended abdomen, and projectile emesis immediately before death. Aspiration was suspected as a cause of his sudden death. In summary, this was a 72-year-old gentleman with early onset neurodegenerative disorder notable for early onset memory, language, and mood dysfunction in the absence of motor impairment consistent with the diagnosis of the semantic variant primary progressive aphasia (svPPA). Expected pathology for svPPA would be underlying TDP-43 deposition which this patient did not have.
